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Thanks to Juan Jose Seguara Fonseca, Laboratorio de Patologia y Citologia Diagnostica (Costa Rica), for contributing this case and most of the discussion. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.

BASIC PATHOLOGY RESEARCH

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Laboratory Investigation is the basic pathology research journal with vast coverage of all biomedical disciplines in the pathology spectrum.

A 43 year old man was seen at the outpatient clinic because of right flank pain and gross hematuria. On an intravenous pyelogram, a smooth, large filling defect was noted in the mid-ureter, accompanied by moderate hydroureter. The kidney and ureter were excised. On opening the ureter, a large, elongated, white solid tumor filled the entire ureteral lumen attached to its wall.

Primary ureteral tumors are rare and account for only 1% of all upper genitourinary tract neoplasms. Benign ureteral lesions are even more rare, representing 20% of all ureteral tumors (1). Nonepithelial benign mesodermal tumors are the rarest, comprising less than 3% of all primary tumors of the ureter. They include fibroepithelial polyps, which are the most common (2), solitary neurofibromas (3) neurofibromas associated with von Recklinghausen disease (4) and sporadic primary hemangiomas (5,6).

Only 12 cases of primary leiomyomas of the ureter have been reported since Leighton's initial report in 1955 (7-18). The patients were usually in the 4th to 5th decades of life, with only two cases in children (9-16). Two thirds of these cases were in males. One case was associated with multiple endocrine neoplasia Type 1 (15). Clinically, most patients presented with hydroureter, hydronephrosis, flank pain with hematuria, ureterocele or blood clot retention (10,14). Most ureteral leiomyomas are located within the ureteral lumen in continuity with the muscle wall, without extension to the renal pelvis.